Pharmacologic treatment of pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare, incurable disease characterized by adverse remodeling of the pulmonary vasculature, leading to increased pulmonary arterial pressures and right ventricular failure. Contemporary pharmacotherapy targets 3 distinct molecular pathways that are abnormal in PAH: deficient production of nitric oxide and prostacyclin, and over production of endothelin. Risk assessment is critical in guiding therapeutic decision making and in disease surveillance following treatment initiation. Patients with more advanced disease are best treated with continuous infusion therapy, while those less symptomatic patients may respond to oral or inhaled therapies. Combination therapy is being increasingly utilized in patients who fail to achieve treatment goals.